Chapter 51 Management of Patients With Immunodeficiency Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Learning Objectives • Compare the different types of primary immunodeficiency disorders and their causes, clinical manifestations, potential complications, and treatment modalities. • Describe the nursing management of the patient with an immunodeficiency. • Identify the essential teaching needs for a patient with an immunodeficiency. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Primary Immunodeficiencies • Usually seen in infants and young children • Manifestations: vary according to type, severe or recurrent infections, failure to thrive or poor growth, positive family history • Potential complications: recurrent, severe, potentially fatal infections; related blood dyscrasias or malignancies • Treatment: varies by type, treatment of infection, pooled plasma or immunoglobulin, GM-CSF or GCSF, thymus graft, stem cell or bone marrow transplant Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Immunodeficiency Disorders • Primary – Genetic – May effect phagocytic function, B cells and/or T cells, or the complement system • Secondary – Acquired – HIV/AIDS – Related to underlying disorders, diseases, toxic substances, or medications Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Management • Monitor for signs and symtoms of infections – Note symptoms of inflammatory response may be blunted • Monitor lab values • Promote good nutrition • Address anxiety, stress, and coping • Strategies to reduce risk of infection – Handwashing and strict aseptic technique – Patient protection and hygiene measures: skin care, promote normal bowel and bladder function, pulmonary hygiene Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Patient Teaching • Signs and symptoms of infection • Medication teaching • Prevention of infection – Handwashing – Avoid crowds and persons with infections – Hygiene and cleaning • Nutrition and diet • Lifestyle modifications to reduce risk • Follow-up care Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Question Is the following statement True or False? Severe combined immunodeficiency disease (SCID) is an disorder involving a complete absence of humoral and cellular immunity resulting from an X-linked or autosomal genetic abnormality. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer True Severe combined immunodeficiency disease (SCID) is an disorder involving a complete absence of humoral and cellular immunity resulting from an X-linked or autosomal genetic abnormality. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Chapter 52 Management of Patients With HIV Infection and AIDS Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Learning Objectives • Describe the modes of transmission of HIV infection and prevention strategies. • Describe the host/HIV interaction during primary infection. • Explain the pathophysiology associated with the clinical manifestations of HIV/AIDS. • Describe the clinical management of patients with HIV/AIDS. • Discuss the nursing interventions appropriate for patients with HIV/AIDS. • Use the nursing process as a framework for care of the patient with HIV/AIDS. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Transmission of HIV • Transmitted by body fluids containing HIV or infected CD4 lymphocytes – Blood, seminal fluid, vaginal secretions, amniotic fluid, and breast milk – Most prenatal infections occur during delivery • Casual contact does not cause transmission • Breaks in skin or mucosa increase risk Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins High-Risk Behaviors • Sharing infected injection equipment • Having sexual relations with infected individuals Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Question Is the following statement True or False? The major means of HIV transmission are unprotected sex and the sharing of injection drug use equipment. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer True The major means of HIV transmission are unprotected sex and the sharing of injection drug use equipment. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Prevention • Standard precautions • Safer sex practices and safer behaviors – Abstain from sharing sexual fluids – Reduce the number of sexual partners to one – Always use latex condoms; if allergic to latex, use non-latex condoms • Do not share drug injection equipment • Blood screening and treatment of blood products Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins HIV Life Cycle • Attachment • Uncoating • DNA synthesis • Integration • Transcription • Translation • Cleavage • Budding Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Structure of HIV-1 Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Life Cycle of HIV-1 Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Stages of HIV Disease • Primary infection • HIV asymptomatic • HIV symptomatic • AIDS Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Primary Infection • AKA acute HIV infection/acute HIV syndrome • Part of CDC category A • Symptoms: none to flu-like syndrome • Window period: lack of HIV antibodies • Period of rapid viral replication and dissemination through the body • Viral set point: balance between amount of HIV and the immune response Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins HIV Asymptomatic • CDC category A • More than 500 CD4+ T lymphpocytes/mm3 • Upon reaching the viral set point, chronic asymptomatic state begins • Body has sufficient immune response to defend against pathogens Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins HIV Symptomatic • CDC category B • 200–499 CD4+ lymphpocytes/mm3 • CD4 T cells gradually fall • The patient develops symptoms or conditions related to the HIV infection, which are not classified as category C conditions • Patients who are once treated for a category B condition are considered category B Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins AIDS • CDC category C • Less than 200 CD4+ lymphocytes/mm3 • As levels drop below 100 cell/mm3 the immune system is significantly impaired • Development of listed conditions Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Treatment • Treatment and protocols are continually evolving • Antiretroviral agents – Nucleoside reverse transcriptase inhibitors (NRTIs) – Non-nucleoside reverse transcriptase inhibitors (NNRTIs) – Protease inhibitors (PIs) – Fusion inhibitors – Use of combination therapy • Management also focuses upon the treatment of specific manifestations and conditions related to the disease Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Question Is the following statement True or False? A fungal infection present in almost all patients with HIV/AIDS is Kaposi’s sarcoma. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer False A fungal infection present in almost all patients with HIV/AIDS is candidiasis, not Kaposi’s sarcoma. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Clinical Manifestations of HIV/AIDS: Respiratory • Pneumocystic carini pneumonia (PCP): – Most common infection – Initial symptoms may be nonspecific and may include nonproductive cough, fever chills, dyspnea, and chest pain – If untreated, progresses to pulmonary impairment and respiratory failure – Treatment: TMP-SMZ or pentamidine, prophylactic TMP-SMZ • Mycobacterium avium complex (MAC) • Tuberculosis Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Clinical Manifestations of HIV/AIDS: GI • Oral candidiasis – May progress to esophagus and stomach – Treatment with Mycelex troches or nystatin, ketoconazole • Diarrhea related to HIV infection or enteric pathogens – Octretide acetate for severe chronic diarrhea • Wasting syndrome – 10% weight loss and chronic diarrhea or chronic weakness and fever with absence of other cause – Protein energy malnutrition – Anorexia, diarrhea, GI malabsorption, and lack of nutrition may contribute Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Clinical Manifestations of HIV/AIDS: Oncologic • Kaposi's sarcoma – Cutaneous lesions, but may involve multiple organ systems – Lesions cause discomfort, disfigurement, ulceration, and potential for infection • B-cell lymphomas Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Lesions of Kaposi’s Sarcoma Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Manifestations of HIV/AIDS: Neurologic • HIV encephalopathy – Progressive cognitive, behavioral, and motor decline – Probably directly related to the HIV infection • Cryptococcus neoformans • Other neurologic disorders • Depression Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Process: The Care of the Patient with HIV/AIDS—Assessment • Assess physical and psychosocial status • Identify potential risk factors: IV drug abuse, risky sexual practices • Immune system function • Nutritional status • Skin integrity • Respiratory status neurologic status • Fluid and electrolyte balance • Knowledge level Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Process: The Care of the Patient with HIV/AIDS- Diagnosis • Impaired skin integrity • Diarrhea • Risk for infection • Activity intolerance • Disturbed thought processes • Ineffective airway clearance • Pain • Imbalanced nutrition • Social isolation • Anticipatory grieving • Deficient knowledge Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Collaborative Problems/Potential Complications • Opportunistic infections • Impaired breathing or respiratory failure • Wasting syndrome • Fluid and electrolyte imbalance • Adverse reaction to medication Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Process: The Care of the Patient with HIV/AIDS—Planning • Goals may include: – Achievement and maintenance of skin integrity – Resumption of usual bowel patterns – Absence of infection – Improved activity tolerance – Improved thought processes – Improved airway clearance – Increased comfort, improved nutritional status – Increased socialization – Expression of grief – Increased knowledge regarding disease prevention and self-care – Absence of complications Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Skin Integrity • Frequent routine assessment of skin and mucosa • Encourage patient to maintain balance between rest and activity • Reposition at least every 2 hours and as needed • Pressure reduction devices • Instruct patient to avoid scratching • Use gentle, nondrying soaps or cleansers • Avoid adhesive tape • Perianal skin care Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Promoting Usual Bowel Pattern • Assess bowel pattern and factors that may exacerbate diarrhea • Avoid foods that act as bowel irritants, such as raw fruits and vegetables, carbonated beverages, spicy foods, and foods of extreme temperatures • Small, frequent meals • Administer medications as prescribed • Assess and promote self-care strategies to control diarrhea Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Activity Intolerance • Maintain balance between activity and rest • Instruction regarding energy conservation techniques • Relaxation measures • Collaboration with other members of the health care team Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Maintaining Thought Processes • Assess mental and neurologic status • Use clear, simple language if mental status is altered • Establish and maintain a daily routine • Orientation techniques • Ensure patient safety and protect from injury • Strategies to maintain and improve functional ability • Instruct and involve family in communication and care Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nutrition • Monitor weight, I&O, dietary intake, and factors that interfere with nutrition • Dietary consult • Control of nausea with antiemetics • Oral hygiene • Treatment of oral discomfort • Dietary supplements • May require enteral feedings or parenteral nutrition Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Decreasing Isolation • Promote an atmosphere of acceptance and understanding • Assess social interactions and monitor behaviors • Allow patient to express feelings • Address psychosocial issues • Provide information related to the spread of infection • Educate ancillary personnel, family, and partners Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Other Interventions • Improving airway clearance – Position in semi-Fowler's or high Fowler’s – Pulmonary therapy; coughing and deep breathing, postural drainage, percussion, and vibration – Ensure adequate rest • Pain – Medications as prescribed – Skin and perianal care Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Question What antiretroviral medication when taken with a highfat/high-caloric meal increases peak plasma concentrations of capsules? A. Delavirdine (Rescriptor) B. Efavirenz (Sustiva) C. Nevirapine (Viramune) Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer B The antiretroviral medication that when taken with a highfat/high-caloric meal increases peak plasma concentrations of capsules is efavirenz (Sustiva). Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Chapter 54 Management of Patients With Autoimmune Disorders •Rheumatoid Arthritis •Lupus •Scleroderma Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Learning Objectives • Explain the pathophysiology of selected autoimmune disorders (diffuse connective tissue disease). • Describe the assessment and diagnostic findings that may be seen in patients with a suspected diagnosis of diffuse connective tissue disease. • Discuss appropriate nursing interventions based on nursing diagnoses and collaborative problems that commonly occur with diffuse connective tissue disease. • Describe the systemic effects of a diffuse connective tissue disease. • Identify modifications in interventions to accommodate changes in patients’ functional ability that may occur with disease progression. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Question Is the following statement True or False? Diarthrodial is bleeding into a joint. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer False Diarthrodial is a joint with two freely movable parts. Hemarthrosis is bleeding into a joint. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Diffuse Connective Tissue Diseases • A group of chronic disorders characterized by diffuse inflammation and degeneration in the connective tissue • Cause is unknown but thought to have an immunologic basis • Characterized by a clinical course of exacerbations and remissions • Includes SLE, scleroderma, polymyositis, and polymyalgia rheumatica Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Rheumatoid Arthritis • More than 100 different disorders • Affect primary the joints, but also muscles, bone, ligament, tendons, cartilage • Classification: – Monoarticular or polyarticular – Inflammatory or noninflammatory Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Characteristic Degenerative Changes— “Degradation” Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Clinical Manifestations • Pain • Joint swelling • Limited movement • Stiffness • Weakness • Fatigue Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Pathophysiology and Associated Physical Signs of Rheumatoid Arthritis Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Patient Assessment and Diagnostic Findings • Health history: include onset of and evolution of symptoms, family history, past health history, and contributing factors • Functional assessment • Arthrocentesis • X-rays, bone scans, CTs, and MRIs • Tissue biopsy • Blood studies Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Interventions • Understanding of the underlying disease process guide; the nurse’s critical thinking to provide interventions • The extent of the disease process, and whether it is localized or more systemic, will also affect nursing activities Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Process: The Care of the Patient with a Rheumatoid Arthritis—Assessment • Health history and physical assessment focus on current and past symptoms, and also include the patient's psychological and mental status, social support systems, ability to participate in daily activities, comply with treatment regimen, and manage self-care Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Process: The Care of the Patient with a Rheumatoid Arthritis—Diagnoses • Acute and chronic pain • Fatigue • Disturbed sleep pattern • Impaired physical mobility • Self-care deficits • Disturbed body image • Ineffective coping Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Collaborative Problems/Potential Complications • Adverse effects of medications Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Nursing Process: The Care of the Patient with a Rheumatoid Arthritis—Planning • Major goals may include: – Relief of pain and discomfort – Relief of fatigue – Promotion of restorative sleep – Increased mobility – Maintenance of self-care – Improved body image – Effective coping – Absence of complications Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Question With which of these diagnoses should the nurse encourage the patient to restrict consumption of foods high in purine? A. Fibromyalgia B. Gout C. Osteoarthritis D. Rheumatoid arthritis Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer B The nurse encourage the patient to restrict consumption of foods high in purine for gout. Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Systemic Lupus Erythematosus (SLE) • AKA Lupus • Incurable autoimmune disease • Cause is unknown • Affects 20-50:100,000 with an annual incidence of 1.6-7.6:100,000 • Onset between the ages of 15 and 45 • Women affected 8 times more often than men Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Risk Factors • Exposure to certain viruses (e.g., Epstein-Barr virus) • Environmental factors (e.g., sunlight, thermal burns) • Genetic factors • Hormonal factors • Certain drugs have been shown to induce SLE – Hydralazine – Procainamide – Isoniazid – Chlorpromazine – Some anti-seizure medications Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Signs and Symptoms/Clinical Presentation • Musculoskeletal symptoms: – Arthralgia – Arthritis – Joint swelling and tenderness – Pain on movement • Cutaneous symptoms: – Malar (butterfly) rash (present in 50% of SLE) Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Signs and Symptoms/Clinical Presentation Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins Teaching Patients Self-Care • Explain the disease and principles of disease management • Medication teaching • Monitoring • Sources of information • Pain management • Joint protection • Self-care with assistive devices • Exercise and relaxation Copyright © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins